Cutaneous T-cell lymphoma

Diagnosis

Types of CTCL

Phases of CTCL

Stages of CTCL

Prognosis

Treatment

Treatment by stage of CTCL

 

Cutaneous T-cell lymphoma (CTCL) is an uncommon type of non-Hodgkin lymphoma (NHL). It accounts for only about 3% of all NHLs. CTCL is a cancer of mature T lymphocytes that mainly affects the skin. Other types of lymphoma can involve the skin, including B-cell and T-cell types of lymphoma, but these are different from CTCL. Most lymphomas of the skin begin in the T lymphocytes. It is also called primary cutaneous T-cell lymphoma.

 

CTCL usually occurs in adults 40–60 years of age. It seems to occur more often in men than in women.

Diagnosis

To diagnose CTCL, doctors remove a small piece of skin from the affected area (biopsy). Different methods can be used to take a skin biopsy. The choice depends on the location and size of the affected area and the type of skin cancer the doctor suspects.

 

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Types of CTCL

CTCL is not a single disease, but a group of different lymphomas that affect the skin. The 2 most common types of cutaneous T-cell lymphomas are mycosis fungoides and Sezary syndrome. With mycosis fungoides, the skin is mainly affected. Sezary syndrome affects both the skin and blood.

Mycosis fungoides

Mycosis fungoides (MF) is the most common form of CTCL. It occurs more often than Sezary syndrome. MF starts in the skin in areas of the body not usually exposed to the sun. It may appear as a scaly, pink or red rash or patches on the skin. Sometimes people do not go to see a doctor about these signs for many months or years. These signs can progress to skin tumours in more advanced cases.

Sezary syndrome

Sezary syndrome (SS) is an advanced form of mycosis fungoides. It affects both the skin and the blood. Sezary syndrome is named after the doctor who first identified it and found that some people with this skin condition also had cancerous lymphocytes in the blood.

 

Large areas of the skin are affected with SS. The skin is often red, itchy and peeling. The entire body can be affected, which is why it is also known as "red man syndrome". Affected areas can be painful. Skin tumours can also appear.

 

Sezary syndrome is often the more aggressive form of CTCL. In addition to the skin and blood, Sezary syndrome can also affect the lymph nodes.

 

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Phases of CTCL

The phases of CTCL vary and may appear in the form of patches, plaques, tumours or ulcers of the skin. In early phases, the lesions are often mistaken for benign skin conditions like psoriasis or eczema.

 

Phase

Description

premycotic (pretumour) phase

small, scaly, red rashes or patches on the skin

rashes can appear anywhere on the body, in areas that are not exposed to the sun

does not cause symptoms and may last for months or years

patch phase

flat, thin, red patches or eczema-like rash on the skin

plaque phase

raised red patches (plaques) on the skin

tumour phase

lumps or nodules (tumours) on the skin

tumours can cause open areas (ulcers), which can become infected

 

Since CTCL usually grows slowly, it may be many years before one phase progresses to the next.

 

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Stages of CTCL

Staging is a way to describe or classify CTCL based on the extent of disease in the body. CTCL is staged based on the type of skin lesions and the extent to which the disease has spread.

 

Stage

Explanation

stage IA

Less than 10% of the skin is covered with patches or plaques.

stage IB

10% or more of the skin is covered with patches or plaques.

stage IIA

Any amount of the skin is covered in patches or plaques.

Lymph nodes are enlarged, but do not contain cancer.

stage IIB

There is one or more raised tumours on the skin.

Lymph nodes may or may not be enlarged and do not contain cancer.

stage III

Almost all of the skin is reddened. There may or may not be patches, plaques or skin tumours.

Lymph nodes may or may not be enlarged and do not contain cancer.

stage IVA

Cancer has spread to the lymph nodes, but not to other organs in the body.

stage IVB

Cancer has spread to other organs in the body, including the blood and bone marrow.

Lymph nodes may be enlarged and may contain cancer.

 

Recurrent CTCL means that the cancer has come back (has recurred) after it has been treated. It may recur in the same location as the original cancer or it may recur in another part of the body (metastatic CTCL).

 

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Prognosis

CTCL is usually a slow-growing (indolent) lymphoma. The prognosis for people with CTCL is based on the extent of disease and how the person responds to treatment. For early stage disease, when the lymphoma is confined to the skin, the prognosis is very favourable. However, if CTCL has spread to lymph nodes or other organs and the disease is more advanced, then the prognosis is not as favourable. Although more advanced stages of CTCL may not be cured, the lymphoma can still be controlled with treatment.

 

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Treatment

Many different types of treatment can be used for CTCL. Treatment can also include measures to relieve itching, such as soothing baths, antihistamines and corticosteroid creams.

Phototherapy or ultraviolet light therapy

Phototherapy involves using ultraviolet light to treat CTCL. This treatment uses 2 kinds of ultraviolet light – ultraviolet A or ultraviolet B.

  • PUVA stands for psoralen (P) and ultraviolet A (UVA) light therapy.
    • PUVA treatment consists of giving a drug called psoralen and then a certain amount of ultraviolet A light is used on the skin. Psoralen makes the skin very sensitive to the effects of UVA light, which helps destroy the lymphoma cells.
    • Psoralen is taken as a pill, usually about 2 hours before the skin is treated with the UVA light.
    • PUVA is effective for treating thick patches and plaques.
    • PUVA treatments are given much the same as a tanning session under a sunlamp.
    • Treatments are given several times (often 3 times) a week at first. When the person responds, then the number of treatments is usually decreased. Treatments may need to be continued on a regular basis for several months (maintenance therapy).
    • PUVA treatment is sometimes called photochemotherapy.
  • Ultraviolet B (UVB) light may also be used. Psoralen is not used with UVB treatment. UVB therapy is effective in treating skin patches or thin plaques. Treatment with UVB phototherapy may also be given several times a week.

 

Phototherapy is used to treat CTCL that isn't very thick and can be used if large areas of skin are affected.

 

Psoralen can make the eyes sensitive to ultraviolet light, so protective sunglasses should be worn. It can also sometimes cause nausea and vomiting. Treatment with ultraviolet light causes the skin to tan. It can also cause premature aging of the skin and increase the risk of skin cancer.

Radiation therapy

External beam radiation therapy may be used to treat CTCL because it is very sensitive to the effects of radiation. Usually, a special type of external beam radiation therapy is used to treat this type of skin lymphoma. Electron beam radiation therapy uses electrons to deliver the radiation, but it is a non-penetrating beam. The radiation does not go through the skin, so it only affects the skin. It does not penetrate deeply enough to affect other organs or the bone marrow.

  • Local external beam radiation therapy may be used if only 1 or 2 small areas of skin are affected. It may also be used to treat patches that remain after PUVA treatment.
  • Total skin electron beam (TSEB) therapy may be used to treat larger areas of skin. TSEB is used if the CTCL is more widespread and skin all over the body needs to be treated. It is usually given only once to treat a person with CTCL, but can sometimes be repeated using reduced doses if CTCL recurs. TSEB can cause a sunburn-like reaction and people may lose their finger nails, toe nails and hair. TSEB requires special equipment and may not be available in all treatment centres.

 

Radiation therapy can also be used in combination with other treatments.

Chemotherapy

Chemotherapy drugs used to treat CTCL are put directly onto the skin (topical chemotherapy), either as an ointment or liquid. Topical chemotherapy is usually used to treat limited disease or early stage CTCL because it is a local therapy. Topical chemotherapy drugs include:

  • mechlorethamine (nitrogen mustard, Mustargen) – used most often
  • carmustine (BiCNU, BCNU)

 

Chemotherapy drugs can also be given as a systemic therapy that circulates throughout the body and destroys cancer cells. Chemotherapy drugs used to treat CTCL can be taken by mouth (orally) or given into the vein (intravenously). Systemic chemotherapy is used to treat CTCL that is more advanced, that has relapsed, or that no longer seems to be responding to other treatments.

 

The most common chemotherapy pills used to treat CTCL are:

  • methotrexate
  • chlorambucil (Leukeran)
  • etoposide (Vepesid, VP-16)

 

Intravenous chemotherapy drugs that may be used to treat CTCL include:

  • fludarabine (Fludara)
  • cladribine (2-CDA, Leustatin)
  • pentostatin (deoxycoformycin, Nipent)
  • pegylated liposomal doxorubicin (Caelyx)
  • gemcitabine (Gemzar)

 

Combinations of drugs may also be used, especially if CTCL has spread to the lymph nodes or other organs, or for people with SS. Chemotherapy may be helpful for people with advanced disease that doesn't respond to other therapies. CHOP or CHOP-like therapies may used to treat CTCL.

  • CHOP – cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone (Deltasone)

Retinoids

Retinoids are drugs that are similar to vitamin A and interfere with cell growth. Retinoids may be applied to the skin or may be taken by mouth (orally). Bexarotene (Targretin) is one retinoid drug that may be used to treat CTCL. Bexarotene comes in a gel form that can be put on the skin. It is used for early stage CTCL with limited skin involvement. It can also be taken as a pill and is used for people with extensive skin involvement or who relapse.

Biological therapy

Biological therapy drugs like interferon alfa (Intron A, Wellferon) may be used to treat CTCL. Interferon alfa is injected under the skin into the fatty tissue (subcutaneously) to help boost the immune response and treat CTCL. It may be used alone or in combination with other treatments, such as PUVA.

 

Denileukin diftitox (Ontak) is a newer drug that is a combination of the biological therapy drug interleukin-2 (Aldesleukin, Proleukin) and the diphtheria toxin. The interleukin finds the CTCL cells and the diphtheria toxin kills them. Researchers have studied denileukin and found it is most useful after other treatments have failed.

 

Alemtuzumab (Campath) is a monoclonal antibody that is being studied in some people with CTCL.

Photopheresis

Photopheresis involves running a person's blood from a vein in their arm through a machine that exposes it to ultraviolet A light. Similar to PUVA treatment, psoralen is used to make the cancerous white blood cells in the blood more sensitive to the effects of UVA light. The treated blood is then returned (reinfused) back into the body. This treatment is used for Sezary syndrome or for progressing CTCL. Photopheresis treatments often need to be repeated several times. Photopheresis may also be called extracorporeal photochemotherapy (ECP).

Surgery

Surgery is occasionally used for some lymphomas of the skin. It is most often used to do a skin biopsy to diagnose and classify a skin lymphoma.

Stem cell transplant

Stem cell transplants (SCTs) are not used very often for people with CTCL.

 

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Treatment by stage of CTCL

Treatment of CTCL depends on how much of the skin's surface is involved (stage). Early stage CTCL may be treated with skin-directed therapies, such as topical chemotherapy or phototherapy (ultraviolet light therapy). These treatments may be used alone or in combination with one another.

 

More extensive disease is treated with electron beam radiation therapy or systemic chemotherapy, biological therapy or systemic retinoid therapy. These treatments are given to help reduce the size of tumours, relieve symptoms and improve the person's quality of life.

Treatment of stage I CTCL

In stage IA CTCL, less than 10% of the skin is covered with patches or plaques. In stage IB, more that 10% of the skin is affected. Treatment options for stage I CTCL include:

  • PUVA – may be given by itself or with interferon alfa
  • radiation therapy to 1 or 2 skin lesions (local radiation therapy) or to all the skin on the body (TSEB)
  • topical chemotherapy
  • biological therapy with interferon alfa – may be given by itself or with topical chemotherapy
  • retinoid therapy
  • systemic chemotherapy

Treatment of stage II CTCL

In stage II CTCL, any amount of the skin is covered in patches or plaques or there is one or more raised tumours on the skin. Lymph nodes may or may not be enlarged and do not contain cancer. Treatment options for stage II CTCL include:

  • PUVA – may be given by itself or with interferon alfa
  • radiation therapy to 1 or 2 skin lesions (local radiation therapy) or to all the skin on the body (TSEB)
  • topical chemotherapy
  • biological therapy with interferon alfa – may be given by itself or with topical chemotherapy
  • retinoid therapy
  • systemic chemotherapy

Treatment of stage III CTCL

In stage III CTCL, almost all of the skin is reddened. There may or may not be patches, plaques or skin tumours. Lymph nodes may or may not be enlarged and do not contain cancer. Treatment options for stage III CTCL include:

  • PUVA – may be given by itself or with interferon alfa or systemic chemotherapy
  • radiation therapy to all the skin on the body (TSEB)
  • radiation therapy as palliative therapy to reduce the size of tumours or relieve symptoms
  • systemic chemotherapy – may be combined with other skin-focussed therapies
  • topical chemotherapy
  • biological therapy with interferon alfa – may be given by itself or with topical chemotherapy
  • retinoid therapy
  • photopheresis

Treatment of stage IV CTCL

In stage IV CTCL, the cancer has spread to the lymph nodes or to other organs in the body. Treatment options for stage IV CTCL include:

  • PUVA – may be given by itself or with interferon alfa or systemic chemotherapy
  • radiation therapy to all the skin on the body (TSEB)
  • radiation therapy as palliative therapy to reduce the size of tumours or relieve symptoms
  • systemic chemotherapy
  • topical chemotherapy
  • biological therapy with interferon alfa – may be given by itself or with topical chemotherapy
  • retinoid therapy
  • photopheresis – may be given by itself or with TSEB

Treatment of recurrent CTCL

Treatment options for recurrent CTCL depend on the extent of the recurrence, past treatment and response to treatment:

  • more TSEB radiation in some cases
  • radiation therapy to bulky tumours or lymph nodes
  • PUVA
  • topical or systemic chemotherapy
  • biological therapy

 

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For more detailed information on specific drugs, go to sources of drug information.

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